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March 12, 2019; 92 (11) Disputes & Debates: Editors' Choice

Editors' note: IgLON5-mediated neurodegeneration is a differential diagnosis of CNS Whipple disease

View ORCID ProfileAriane Lewis, Steven Galetta
First published March 11, 2019, DOI: https://doi.org/10.1212/WNL.0000000000007086
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Editors' note: IgLON5-mediated neurodegeneration is a differential diagnosis of CNS Whipple disease
Ariane Lewis, Steven Galetta
Neurology Mar 2019, 92 (11) 543; DOI: 10.1212/WNL.0000000000007086

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In “IgLON5-mediated neurodegeneration is a differential diagnosis of CNS Whipple disease,” Morales-Briceño et al. described a case of anti-IgLON5 disease presenting as cold intolerance, myorhythmia, myoclonus, dysarthria, hypersalivation, dysphagia, sleep apnea, and mildly impaired cognition mimicking CNS Whipple disease. Drs. Bally and Lang report that the presentation in this case was similar to cases of Whipple disease in the literature; however, palatal involvement of the myorhythmia—which was present in the case reported by Morales-Briceño et al.—is rare in Whipple disease, and the cranial myorhythmia in Whipple disease is associated with a vertical supranuclear gaze palsy, which was not present in the case reported by Morales-Briceño et al. Morales-Briceño et al. respond that while the absence of vertical gaze palsy made Whipple disease less likely, such gaze palsies can occur in a certain percentage of patients with IgLON5-mediated neurodegeneration. Both Morales-Briceño et al. and Drs. Bally and Lang agree that the distinction between IgLON5-mediated neurodegeneration and Whipple disease can be challenging.

In “IgLON5-mediated neurodegeneration is a differential diagnosis of CNS Whipple disease,” Morales-Briceño et al. described a case of anti-IgLON5 disease presenting as cold intolerance, myorhythmia, myoclonus, dysarthria, hypersalivation, dysphagia, sleep apnea, and mildly impaired cognition mimicking CNS Whipple disease. Drs. Bally and Lang report that the presentation in this case was similar to cases of Whipple disease in the literature; however, palatal involvement of the myorhythmia—which was present in the case reported by Morales-Briceño et al.—is rare in Whipple disease, and the cranial myorhythmia in Whipple disease is associated with a vertical supranuclear gaze palsy, which was not present in the case reported by Morales-Briceño et al. Morales-Briceño et al. respond that while the absence of vertical gaze palsy made Whipple disease less likely, such gaze palsies can occur in a certain percentage of patients with IgLON5-mediated neurodegeneration. Both Morales-Briceño et al. and Drs. Bally and Lang agree that the distinction between IgLON5-mediated neurodegeneration and Whipple disease can be challenging.

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